Child dementia Even children can develop dementia

A new study on causes of childhood dementia is being prepared by pharmacologist Christian Grimm, and he has now received the NCL Research Award. It's about hereditary neuronal ceroid lipofuscinosis, which is currently incurable.

A deadly disease
In this form, the cell organelles do not work as they should. Damage in various proteins of these lysosomes causes several variants of the disease, but so far, the researchers knew little about how these proteins act.

Even children can suffer from dementia. Image: Brian Jackson - fotolia

Blindness, epilepsy and decay
The nerve cells of those affected die, they lose sight to blindness, they suffer from disturbance of consciousness, the musculoskeletal system stops functioning, they twitch uncontrollably and so far they inevitably die, because the functions that maintain the organism fail. Increased mortality from epilepsy alone is only one reason why these children die early.

The CLN3 protein
Grimm is now trying to decode the protein CLN3. This protein causes the disease, which begins at primary school age and kills the nerve cells - in the end, the patient dies. CLN3 occurs in the lysosomes that degrade biomolecules. Grimm suspects CLN3 as an essential channel for ions and calcium. He now examines these functions with the so-called patch-clamp method.

The patch-clamp technique
Patch-Clamp allows to observe CLN3 proteins in their electrical behavior. The scientists suck on a part of the membrane with a micropipette, put on a microelectrode and send current into the ion channels. This will show you whether the channel is being used or not.

Grimm wants to show if CLN3 is an ion channel and also recognize the molecules that activate CLN3. For this, the researchers additionally need a systematic screening.

New agents for therapy
CLN3 is often absent in those affected. Grimm therefore wants to find substances that can replace the protein. Some substances already discovered the scientists who are eligible. (Dr. Utz Anhalt)